ASSESSMENT OF QOL IN PATIENTS WITH NEUROFIBROMATOSIS 1 We carried out a cross-sectional study to evaluate the impact of NF1 on QoL

in an Italian cohort. Recruitment took place in a single Italian Centre, the Dermatological and Neurofibromatosis out-patients Clinic of the University of Rome “La Sapienza”. From February 2005 to September 2005, 129 patients with NF1 were enrolled. In conclusion For all domains of SF-36 profile, patients with NF1 had lower scores than the control sample

QoL was measured using the SF-36, a general health questionnaire, and the Skindex, a specific QoL questionnaire designed to assess the QoL in patients with skin diseases. For the clinical assessment, the Ricciardi and Ablon scales were used to evaluate the severity of the disease and the physical disfigurements. The Ricciardi scale evaluates the severity of the diseases on a scale from 1 to 4. Briefly, grade 1, or minimal NF1, means the presence of few features of NF1; grade 2, or mild NF1, indicates obvious but not healththreatening features. In grade 3, or moderate NF1, there is an equilibrium between health and well-being, but it is still manageable. Grade 4, or severe NF1, indicates health and well-being “seriously” compromised in a permanent and “unmanageable way”. The Ablon scale evaluates the appearance of the disease ratings the appearance of the person fully dressed from mild (grade 1) to severe (grade 3).

Statistical analysis Scaled scores of participants in different clinical groups were compared using Wilcoxon test and variance analysis. Frequency distribution, tabulation, stratified analysis and multiple linear regression controlling for sex, age, severity and appearance were performed with STATA and EPI-INFO software.

Results The SF-36 results of the 129 patients with NF1 were compared to those of an healthy sample of 2031 subjects representative of the general population. For all domains of SF-36 profile, patients with NF1 had lower scores than the control sample (Table 4.1.1).

Table 4.1.1 Quality of life of adult patients measured with SF-36 compared with a normative sample representative of the Italian population.
NF1 Patients (n=129) 79.2± 25.9 75.1± 33.9 69.1±27.3 57.5±19.2 60.3±20.2 68.6±26.4 61.6±39.7 64.4±20.8 Health Sample (n=2031) 84.4±23.9 77.9±36.1 73.7±27.2 65.9±22.2 61.8±20.8 77.1±23.5 75.7±23.5 66.7±20.9 P 0.03 0.20 0.03 0.00 0.15 0.00 0.00 0.1

Physical function Role-physical Bodily pain General health perception Vitality Social functioning Role-emotional Mental Health

The Skindex quality of life questionnaire confirmed that NF1 has an impact in all aspects of Skin-diseasespecific QoL, with the emotional aspect being the most affected (Figure 4.1.1).

Figure 4.1.1 Skindex quality of life scores for patients with NF1
Male 40 Female

30 mean score

20

10

0 Emotions Physical symtoms Functioning

Patients with more visible NF1, accordingly to the Ablon Index, reported more effects on their skin-diseasespecific QoL (Table 4.1.2)

Table 4.1.2 Visibility Measured with Ablon Index and Skindex in Patients with NF1
Skindex Characteristic Emotions Physical Symptoms Functioning Ablon Index Grade 2 (n=52) 32.6±19.6 21.8±17.7 17.7±16.3

Grade I (n=38) 15.6±12.9 10.5±12.2 7.5±10.6

Grade 3 (n=27) 39.5±23.2 23.8±21.3 28.4±24.8

P 0.000 0.001 0.0001

These findings (Table 4.1.2) suggest that Skindex-29 measures skin-disease-related aspects more specifically and accurately than the generic measure. This ability of Skindex to measure the specific skin-disease-related health characteristics may be particular important when a more specific tool is desired and to determinate whether the measure might supplement the clinical assessment.

Our study showed an association between the educational level and different health domains of the SF 26 (Table 4.1.3): higher levels of education have more positive perception on QoL.

Table 4.1.3 General Health Quality of Life Measured with SF-36 and educational level
Educational level SF-36 Characteristics Physical function Role-physical Bodily pain General health perception Vitality Social functioning Role-emotional Mental Health Mental Composite Score Physical Composite Score Primary school 64.2±23.8 42.8±47.2 53.1±28.9 43.8±21.6 46.4±15.1 71.5±26.6 38.1±40.5 53.1±21.9 40.0±9.8 40.5±8.1 Secondary school 75.3±27.3 70.8±34.5 64.7±26.9 56.8±20.1 60.1±21.5 64.8±28.4 57.2±41.5 60.9±24.2 43.1±12.7 47.4±10.2 High School 84.1±25.4 82.2±28.8 75.6±27.0 58.4±18.2 61.8±19.5 74.0±23.5 67.3±37.6 69.3±16.7 45.8±10.2 50.2±8.8 University degree 98.1.1±3.7 96.4±9.4 77.8±21.2 72.4±19.7 66.4±16.2 68.7±20.0 58.2±46.3 69.7±12.8 42.1±11.5 56.0±5.7 P 0.002 0.02 0.04 0.09 0.13 0.47 0.27 0.20 0.43 0,008

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